Pediatric Restrictive Cardiomyopathy Medication

Updated: Sep 18, 2019
  • Author: Kimberly Y Lin, MD; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Medication

Medication Summary

Therapy for idiopathic restrictive cardiomyopathy (RCM) is limited to symptomatic treatment and is often ineffective in improving outcome.

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Diuretic agents

Class Summary

Diuretic agents promote excretion of water and electrolytes by the kidneys. Treatment with diuretics may improve symptoms of venous congestion. However, these agents should be used with caution, because some patients require high venous filling pressures to maintain adequate cardiac output.

Furosemide (Lasix)

Furosemide is a loop diuretic that blocks the sodium-potassium-chloride transporter and works primarily on thick ascending limb of the loop of Henle. It also inhibits sodium and chloride absorption from the proximal and distal tubules.

Chlorothiazide (Diuril)

Chlorothiazide is a thiazide diuretic that blocks the electroneutral sodium-chloride transporter.

Metolazone (Zaroxolyn)

Metolazone is a quinazoline diuretic with properties similar to those of thiazide diuretics. It inhibits sodium resorption at the cortical diluting site and the proximal convoluted tubule.

Spironolactone (Aldactone)

Spironolactone is an aldosterone antagonist that spares potassium. It competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

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Anticoagulant

Class Summary

Some authors advocate the use of anticoagulation, antiplatelet agents, or both in children with RCM because of due to the high reported incidence of thromboembolic events in several small case series. If chosen, anticoagulation agents should be carefully administered with close supervision of coagulation parameters.

Heparin

Heparin augments the activity of antithrombin III and prevents conversion of fibrinogen to fibrin. It does not actively lyse but is able to inhibit further thrombogenesis. It prevents reaccumulation of clot after spontaneous fibrinolysis.

Warfarin (Coumadin)

Warfarin interferes with hepatic synthesis of vitamin K–dependent coagulation factors. It is used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders.

Enoxaparin (Lovenox)

Enoxaparin is a low-molecular-weight heparin that differs from unfractionated heparin by having a higher ratio of antifactor Xa to antifactor IIa.

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Antiplatelet Agent

Class Summary

Low-dose aspirin is the predominant platelet aggregation inhibitor used in children, although only limited comparative data regarding effective antiplatelet doses in pediatric populations are available.

Aspirin (Bayer Aspirin Extra Strength, Ecotrin, Aspercin)

Aspirin is a stronger inhibitor of both prostaglandin synthesis and platelet aggregation than other salicylic acid derivatives are. The acetyl group is responsible for inactivation of cyclooxygenase via acetylation. Aspirin irreversibly inhibits platelet aggregation by inhibiting platelet cyclooxygenase. This, in turn, inhibits conversion of arachidonic acid to prostaglandin I2 (a potent vasodilator and inhibitor of platelet activation) and thromboxane A2 (a potent vasoconstrictor and platelet aggregate). Platelet-inhibition lasts for the life of the cell (approximately 10 days).

Aspirin may be used in low doses to inhibit platelet aggregation and improve complications of venous stasis and thrombosis.

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